Atypical Presentation Of Disease Research Articles

Atypical presentation of adult celiac disease: myocarditis and liver abnormality

Atypical presentation of adult celiac disease: myocarditis and liver abnormality

Lipoma arborescens: Comparison of typical and atypical disease presentations

To determine whether the aetiology differed between typical cases of lipoma arborescens with unilateral knee involvement and atypical cases involving joints other than the knee, polyarticular disease, and disease outside of the knee joint. Cases of lipoma arborescens involving the knee joint were evaluated for the distribution of the disease and severity of degenerative arthritis. Joints other than the knee were evaluated for the presence and severity of degenerative arthritis, and the distribution was classified as either intra-articular, extra-articular, or both. Clinical history was reviewed for patient age at presentation, a history of inflammatory arthritis, diabetes mellitus, and known steroid use. Fisher's exact test was used to determine whether there was a statistically significant difference between typical and atypical presentations of the disease. Lipoma arborescens was identified in 45 joints in 39 patients. Twenty-eight patients were classified as "typical" and 11 patients had "atypical" disease. There was no significant difference in age at presentation, presence of degenerative arthritis, or known inflammatory arthritis when comparing typical and atypical presentations of the disease. Twenty-eight percent of patients in the present study had atypical presentation of lipoma arborescens with multifocal lipoma arborescens or disease in joints other than the knee. There was no significant difference in age at presentation, presence of degenerative arthritis, or known inflammatory arthritis when comparing typical and atypical presentations of the disease. Of the 39 patients, only three had no evidence of degenerative arthritis, which suggests that many cases of lipoma arborescens are secondary to chronic reactive change in association with degenerative arthritis.

Page kidney phenomenon secondary to an atypical presentation of Erdheim-Chester disease.

A 50-year-old man with a 6-month history of arthralgias, recurrent pericardial and pleural effusions presented to the nephrology clinic with uncontrolled hypertension and elevated creatinine. No proteinuria or hematuria was noted. Computed tomography (CT) scan of the abdomen showed a hypodense tissue encasing both kidneys (Figure 1A). Tissue biopsy revealed a fibroproliferative process, dense fibrosis and focal collections of histiocytes (Figure 1B). These histiocytes had a dense amphophilic cytoplasm, but were not foamy. Given our clinical impression, specific immunostains were performed. Histiocytes stained positive for CD68 (Figure 1C) and factor XIIIa, and negative for CD1a, Alk-1 and S-100. Findings were consistent with Erdheim-Chester disease (ECD) and the patient underwent a trial of interferon therapy with minimal improvement and subsequently imitinab therapy with some stabilization of symptoms. Fig. 1: (A) CT image of the abdomen showed a dense collection encasing the kidneys bilaterally, and inset shows the positron emission tomography scan. (B) Hematoxylin and eosin stain of the tissue biopsy revealed a fibroproliferative process, and focal collections ... ECD is a systemic, infiltrative disease, characterized classically by foamy histiocytes, with immunohistochemical characteristics different from those of Langerhans cell (LC) histiocytosis [1]. LC expresses proteins CD1a and S100 and demonstrates the presence of Birbeck granules [1, 2]. ECD is composed of histiocytes that express evidence of phagocytic differentiation, hence stain positive for CD68, but lack proteins CD1a and S100 and Birbeck granules [1, 2]. ECD shows a tropism for connective, adipose and perivascular tissues, almost always involves bone with characteristic osteosclerosis of long bones and can present with potentially life-threatening complications, such as heart failure and tamponade [1, 2]. Renal and perirenal involvement was found in 29% of patients [2]. The morphologic and clinical presentation is different from IgG4-related disease that consists of a lymphoplasmacytic infiltrate [3]. The treatment of ECD usually includes steroids and immunomodulating medications, and surgical decompression has been reported to preserve renal function [4]. Page kidney phenomenon is the extrinsic compression of the renal parenchyma leading to hypertension, via activation of the renin–angiotensin–aldosterone system [5]. Often caused by hemorrhage and malignancies, in our patient it was from the infiltration around the kidneys.

Hookworm With Hypereosinophilia: Atypical Presentation of a Typical Disease

We describe a 55-year-old man returning from the Philippines infected with a hookworm, the novel bacterium Laribacter hongkongensis, and a Blastocystis hominis and presenting with both gastrointestinal and neurological symptoms. The high eosinophilia caused by the hookworm infection resulted in both gastrointestinal and neurological symptoms, resembling a hypereosinophilic syndrome.

Celiac Disease Patients Presenting With Anemia Have More Severe Disease Than Those Presenting With Diarrhea

Anemia is considered to be an atypical or silent presentation of celiac disease, compared with the classic presentation with diarrhea. However, little information is available about how these groups compare in terms of disease severity. We compared the severity of celiac disease between patients who present with anemia vs those who present with diarrhea. The study cohort was selected from a database of patients with celiac disease who were evaluated at a tertiary referral center between 1990 and 2011. Severity of celiac disease was assessed by the degree of villous atrophy and clinical and serologic parameters. Patients were compared according to mode of presentation and sex. Multivariable analyses, adjusting for age and sex, were conducted to assess the association between the mode of celiac disease presentation and cholesterol level, bone density, severity of villous atrophy, erythrocyte sedimentation rate, and level of anti-tissue transglutaminase. Of 727 patients, 77% presented with diarrhea and 23% with anemia (92% iron deficient). On multiple regression analysis, presentation with anemia was associated with lower levels of total cholesterol (P= .02) and high-density lipoprotein (P= .002) and a higher erythrocyte sedimentation rate (P= .001) and level of anti-tissue transglutaminase (P= .01). Presentation with anemia was associated with lower level of cholesterol only in women. Anemic patients were more than 2-fold more likely to have severe villous atrophy and a low bone mass density at the time they were diagnosed with celiac disease than patients who presented with diarrhea. Celiac disease patients who present with anemia have more severe disease than those who present with diarrhea. There also appear to be sex-specific differences with regard to the association between anemia and the different features of celiac disease.

Présentation cutanée atypique d’une maladie des griffes du chat

Herein we report a case of cat scratch disease on account of its atypical presentation.A 21-year-old woman presented erythema nodosum associated with painful bilateral inguinal adenopathy, odynophagia, joint pain and evening urticaria in a setting of impaired general condition. Initial serological testing for Bartonella henselae was negative. PCR for Bartonella henselae performed on an adenectomy fragment was positive. A favourable outcome was achieved with azithromycin.This case shows an atypical and severe presentation of cat scratch disease and raises the problem of sensitivity of serotyping.

A case of isolated middle cerebral artery stenosis with hemichorea and moyamoya pattern collateralization.

Isolated middle cerebral artery (MCA) stenosis in young patients with no other medical condition may be a unique pathologic entity with a benign long-term course. Generally, moyamoya disease shows a progression of stenosis from internal cerebral artery (ICA) to other intracranial vessel. A 26-year-old woman was admitted for choreic movements of the right arm and leg. Brain magnetic resonance imaging showed no stroke. Conventional angiography revealed 48% stenosis of the left M1 without ICA stenosis. Single photon emission computed tomography revealed perfusion asymmetry after acetazolamide injection, suggesting decreased uptake in the left basal ganglia and the cerebral cortex. Her hemichorea was mildly decreased with risperidone. One year later, follow-up angiography showed complete occlusion of the left M1 with neovascularization suggestive of moyamoya disease. The patient underwent bypass surgery and her hemichorea disappeared. This may be an atypical presentation of moyamoya disease. The bypass surgery was an effective measure for restoring the vascular insufficiency and, resultantly, controlling her hemichorea.

Herpesviruses and the microbiome

The focus of this article will be to examine the role of common herpesviruses as a component of the microbiome of atopic patients and to review clinical observations suggesting that atopic patients might be predisposed to more severe and atypical herpes-related illness because their immune response is biased toward a TH2 cytokine profile. Human populations are infected with 8 herpesviruses, including herpes simplex virus HSV1 and HSV2 (also termed HHV1 and HHV2), varicella zoster virus (VZV or HHV3), EBV (HHV4), cytomegalovirus (HHV5), HHV6, HHV7, and Kaposi sarcoma-associated herpesvirus (termed KSV or HHV8). Herpesviruses are highly adapted to lifelong infection of their human hosts and thus can be considered a component of the human "microbiome" in addition to their role in illness triggered by primary infection. HSV1 and HSV2 infection and reactivation can present with more severe cutaneous symptoms termed eczema herpeticum in the atopic population, similar to the more severe eczema vaccinatum, and drug reaction with eosinophilia and systemic symptoms syndrome (DRESS) is associated with reactivation of HSV6 and possibly other herpesviruses in both atopic and nonatopic patients. In this review evidence is reviewed that primary infection with herpesviruses may have an atypical presentation in the atopic patient and conversely that childhood infection might alter the atopic phenotype. Reactivation of latent herpesviruses can directly alter host cytokine profiles through viral expression of cytokine-like proteins, such as IL-10 (EBV) or IL-6 (cytomegalovirus and HHV8), viral encoded and secreted siRNA and microRNAs, and modulation of expression of host transcription pathways, such as nuclear factor κB. Physicians caring for allergic and atopic populations should be aware of common and uncommon presentations of herpes-related disease in atopic patients to provide accurate diagnosis and avoid unnecessary laboratory testing or incorrect diagnosis of other conditions, such as drug allergy or autoimmune disease. Antiviral therapy and vaccines should be administered promptly when indicated clinically.

Frontal presentation of Alzheimer's disease: a series of patients with biological evidence by CSF biomarkers.

Besides its typical amnesic presentation, focal atypical presentations of Alzheimer's disease (AD) have been described in neuropathological studies. These phenotypical variants of AD (so-called "atypical AD") do not follow the typical amnestic pattern and include non-amnestic focal cortical syndromes, such as posterior cortical atrophy and frontal variant AD. These variants exhibit characteristic histological lesions of Alzheimer pathology at post-mortem exam. By using physiopathological markers, such as cerebrospinal fluid markers, it is now possible to establish in vivo a biological diagnosis of AD in these focal cortical syndromes. We report a series of eight patients who were diagnosed with behavioural variant frontotemporal dementia based on their clinical, neuropsychological and neuroimaging findings, while CSF biomarkers showed an AD biological profile, thus supporting a diagnosis of frontal variant of AD.

An Atypical Case Presentation of Adult-Onset Leigh's Disease (P07.206)

An Atypical Case Presentation of Adult-Onset Leigh's Disease (P07.206)

Atypical presentations of dengue disease in the elderly visiting the ED

ObjectiveThe objective was to compare the clinical characteristics of elderly and young adult patients with dengue in the emergency department (ED). MethodsDemographic characteristics, clinical presentation, disease severity, laboratory characteristics, and outcomes were analyzed prospectively as a case-control study. ResultsOf the 193 adults with serologically confirmed dengue disease in 2007, 31 (16.1%) were elderly patients (aged ≥65) and 162 were young adults (aged <65). More dengue hemorrhagic fever (12.9% vs 2.5%, P = .02), a longer ED stay (13.3 vs 8.6 hours, P = .004), a longer hospital stay (7.4 vs 3.4 days, P < .001), a higher Simplified Acute Physiology Score II in the ED (29.7 vs 17.4, P < .001), and a higher rate of at least 1 comorbidity (61.8 vs 22.8%, P < .001) were found in the elderly. However, the length of the intensive care unit stay (elderly 0.7 vs young adults 0.3 day, P = .47) and the 14-day mortality rate (0% vs 0.6%, P = 1.00) were similar. Of note, in terms of clinical presentations of dengue in the ED, there were more elderly patients with isolated fever (41.9% vs 17.9%, P = .003) and fewer with typical presentation (41.9% vs 75.9%, P = <.001) than there were young adults. ConclusionsThe present study found a higher number of atypical presentations, a longer hospitalization, and a higher degree of clinical illness in elderly patients with dengue.

A case presentation of a fatal dengue myocarditis showing evidence for dengue virus-induced lesion

Dengue is a prevalent arthropod-borne viral disease in tropical and subtropical areas of the globe. Dengue clinical manifestations include asymptomatic infections; undifferentiated fever; dengue fever, which is characterized by fever, headache, retroorbital pain, myalgia, and arthralgia; and a severe form of the disease denominated dengue haemorrhagic fever/dengue shock syndrome, characterized by haemoconcentration, thrombocytopenia, and bleeding tendency. However, atypical manifestations, such as liver, central nervous system, and cardiac involvement, have been increasingly reported. We report an atypical and rare presentation of dengue disease marked by a dramatic and fatal cardiogenic shock due to acute myocarditis. Histopathological analysis of heart tissue showed several multifocal areas of muscle necrosis and intense interstitial oedema associated with clusters of virus particles inside the cardiomyocytes and in the interstitial space, providing evidence of a possible direct action of dengue virus on myocardium.

Facilitators and barriers to safe emergency department transitions for community dwelling older people with dementia and their caregivers: A social ecological study

BackgroundInternationally, older adults visit the ED at a rate higher than other age groups. Little attention has been given to ED care for older people with dementia, although concern for such care is growing with the increasing number of individuals worldwide affected by this significant disabling problem. It is critical to understand ED transitional processes and consequences because the complexity of dementia care poses multiple challenges to optimizing safety, effectiveness and quality of care during admission, assessment, and treatment in this setting. MethodUsing an interpretive, descriptive exploratory design with three iterative, interrelated phases, we conducted interviews, created a photographic narrative journal (PNJ), and finally held photo elicitation focus groups to identify factors that facilitate or impede safe transitional care for community dwelling older adults with dementia in two Canadian emergency departments, and to identify practice solutions for nurses. We purposively sampled to recruit ten older adult-family caregiver dyads, ten ED RNs, and four Nurse Practitioners. Data were analyzed using constant comparative analysis. ResultsFour interconnected reinforcing consequences emerged from our analysis: being under-triaged; waiting and worrying about what was wrong; time pressure with lack of attention to basic needs; and, relationships and interactions leading to feeling ignored, forgotten and unimportant. Together these consequences stem from a triage system that does not recognize atypical presentation of disease and illness. This potentiated a cascade of vulnerability in older people with dementia and their caregivers. Nurses experienced time pressure challenges that impeded their ability to be responsive to basic care needs. ConclusionsIn an aging population where dementia is becoming more prevalent, the unit of care in the ED must include both the older person and their family caregiver. Negative reinforcing consequences can be interrupted when nurses communicate and engage more regularly with the older adult-caregiver dyad to build trust. System changes are also needed to support the ability of nurses to carry out best practices.

Poster 225 Mid-Back Pain in a 28-Year-Old Male Rower Presenting to the Sports Medicine Clinic: An Atypical Presentation of Cushing's Disease. A Case Report

Poster 225 Mid-Back Pain in a 28-Year-Old Male Rower Presenting to the Sports Medicine Clinic: An Atypical Presentation of Cushing's Disease. A Case Report

Uncommon Presentation of an Uncommon Condition

Purpose: A 66-year-old male who presented with increasing fatigue, weight loss, anorexia, abdominal pain, and confusion. He started having emesis with streaks of blood on day of admission. On physical exam he was only oriented to person and place and had right sided abdominal tenderness. Stool was positive for occult blood. Blood tests were significant for anemia and hypercalcemia. Computed tomography (CT) scan revealed right renal mass extending into the IVC and expanding the duodenum at the junction of the first and second part. Esophagogastroduodenoscopy (EGD) showed a friable infiltrating mass in the lateral wall of the duodenum. Duodenal biopsy was suggestive of renal cell carcinoma, clear cell type. Tumor burden was prohibitive for surgical intervention, and he was started on weekly Temsirolimus. After 4 cycles of chemotherapy he decided to receive only palliative care. Duodenal metastasis from renal cell carcinoma (RCC) origin is very uncommon. This case report demonstrates the variable presentations of metastatic RCC with duodenal involvement. Despite a large mass at the duodenal wall, he did not have classic symptoms of small bowel obstruction. Moreover, bleeding from this mass manifested as blood streaked emesis in contrast to overt hematemesis that has been classically reported. He also lacked lower extremity and scrotal edema despite tumor infiltration of the IVC. This case underscores the importance to apply fundamental principles of disease pathogenesis and gross anatomy to atypical presentations of disease manifestations.Figure: [914] Computed tomography scan showing duodenum being expanded by tumor mass (arrow) and infrahepatic IVC with a tumor thrombus (arrow head). EGD picture showing friable mass that is oozing blood and invading lateral wall of the duodenum.Figure: [914] Histopathology; biopsies from the duodenal mass revealed mucosal ulceration with a submucosal mass rich in vasculature (A) and clear cytoplasm (B). The morphology is suggestive of renal cell carcinoma, clear cell type and it is confirmed by positive immunohistochemical stains for Vimentin (C) and PAX8, a nephritic-lineage transcription factor (D).

TF-5 Cases in Geriatric Emergency Medicine: An Integrated Simulation Experience

TF-5 Cases in Geriatric Emergency Medicine: An Integrated Simulation Experience

Isolated Neonatal Seizures: Adenylosuccinase Deficiency is Another Cause!

I read with great interest the very useful review “Isolated Neonatal Seizures: When to Suspect Inborn Errors of Metabolism” by Ficicioglu and Bearden [ [1] Ficicioglu C. Bearden D. Isolated neonatal seizures: When to suspect inborn errors of metabolism. Pediatr Neurol. 2011; 45: 283-291 Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar ]. I would like to add adenylosuccinase deficiency to their causes for suspicion. This genetic, mainly neurologic disorder of the de novo purine synthesis was first reported in 1984 [ [2] Jaeken J. van den Berghe G. An infantile autistic syndrome characterized by the presence of succinylpurines in body fluids. Lancet. 1984; 2: 1058-1061 PubMed Google Scholar ]. Most of the approximately 60 reported patients demonstrate variable associations of moderate to severe psychomotor retardation, epilepsy after their first years, autistic features, and growth retardation [ [3] van den Berghe G. Vincent M.-F. Marie S. Disorders of purine and pyrimidine metabolism. in: Saudubray J.-M. van den Berghe G. Walter J.H. Inborn metabolic diseases: Diagnosis and treatment. 5th ed. Springer-Verlag, Berlin2012: 499-518 Crossref Scopus (11) Google Scholar ]. However, a subgroup of patients presents with intractable seizures beginning within the first days or weeks of age [ [4] Mouchegh K. Zikánová M. Hoffmann G.F. et al. Lethal fetal and early neonatal presentation of adenylosuccinate lyase deficiency: Observation of 6 patients in 4 families. J Pediatr. 2007; 150: 57-61 Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar ]. Often severe hypotonia is associated such as seen, e.g., in nonketotic hyperglycinemia and γ-aminobutyric acid transaminase deficiency, as mentioned by Ficicioglu and Bearden [ [1] Ficicioglu C. Bearden D. Isolated neonatal seizures: When to suspect inborn errors of metabolism. Pediatr Neurol. 2011; 45: 283-291 Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar ]. These seizures can lead to death or severe mental retardation. In some patients, intrauterine growth retardation, microcephaly, fetal hypokinesia, and a lack of fetal heart rate variability have also been observed. Adenylosuccinase catalyzes two steps in purine synthesis: the conversion of succinyl aminoimidazole carboxamide ribotide to aminoimidazole carboxamide ribotide (in the de novo pathway), and the conversion of adenylosuccinate to adenosine monophosphate. Its deficiency results in the accumulation in body fluids of succinyl purines, i.e., succinyl aminoimidazole carboxamide riboside and succinyl adenosine (the dephosphorylated substrates of the enzyme). A modified Bratton-Marshall (colorimetric) test performed on urine can be used as a screening test, but some medications such as sulfonamides cause a false-positive result. A final diagnosis requires high-pressure liquid chromatography with ultraviolet light detection, or high-pressure liquid chromatography with electrospray ionization tandem mass spectroscopy. Isolated Neonatal Seizures: When to Suspect Inborn Errors of MetabolismPediatric NeurologyVol. 45Issue 5PreviewNeonatal seizures are common, and often comprise the first clinical indicator of central nervous system dysfunction. Although most neonatal seizures are secondary to processes such as hypoxic-ischemic injury, infection, or cortical malformations (which are readily identifiable through routine testing and imaging), seizures secondary to inborn errors of metabolism can be much more difficult to diagnose, and thus a high index of suspicion is required. The early diagnosis of inborn errors of metabolism is crucial, considering that many can receive effective treatment (e.g., dietary supplementation or restriction) with favorable long-term outcomes. Full-Text PDF ResponsePediatric NeurologyVol. 47Issue 3PreviewWe thank Dr. Jaaken for his insightful discussion of adenylosuccinase deficiency as a cause of neonatal seizures. Our review was intended to offer an overview of metabolic disorders presenting with isolated neonatal seizures, i.e., neonatal seizures not associated with other characteristic abnormalities. Dr. Jaaken is correct in observing that neonatal seizures may constitute an atypical presentation of this rare disease, with six cases reported in the literature [1]. However, the severe form of adenylosuccinase deficiency that presents with neonatal seizures is often accompanied by severe hypotonia, intrauterine growth retardation, and magnetic resonance imaging abnormalities that are variable but that generally include dysmyelination [2]. Full-Text PDF

Response

We thank Dr. Jaaken for his insightful discussion of adenylosuccinase deficiency as a cause of neonatal seizures. Our review was intended to offer an overview of metabolic disorders presenting with isolated neonatal seizures, i.e., neonatal seizures not associated with other characteristic abnormalities. Dr. Jaaken is correct in observing that neonatal seizures may constitute an atypical presentation of this rare disease, with six cases reported in the literature [ [1] Mouchegh K. Zikánová M. Hoffmann G.F. et al. Lethal fetal and early neonatal presentation of adenylosuccinate lyase deficiency: Observation of 6 patients in 4 families. J Pediatr. 2007; 150: 57-61 Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar ]. However, the severe form of adenylosuccinase deficiency that presents with neonatal seizures is often accompanied by severe hypotonia, intrauterine growth retardation, and magnetic resonance imaging abnormalities that are variable but that generally include dysmyelination [ [2] Jurecka A. Jurkiewicz E. Tylki-Szymanska A. Magnetic resonance imaging of the brain in adenylosuccinate lyase deficiency: A report of seven cases and a review of the literature. Eur J Pediatr. 2012; 171: 131-138 Crossref PubMed Scopus (19) Google Scholar ]. In addition, a characteristic facial dysmorphism has been described [ [3] Holder-Espinasse M. Bourrouillou G. Ceballos-Picot I. et al. Towards a suggestive facial dysmorphism in adenylosuccinate lyase deficiency?. J Med Genet. 2002; 39: 440-442 Crossref PubMed Scopus (12) Google Scholar ]. We agree that screening for disorders of purine metabolism, including adenylosuccinase deficiency, is indicated in the evaluation of neonatal seizures without a clear etiology, and cerebrospinal fluid succinyl adenosine and urine purine panels, which are clinically available, should be added to our diagnostic algorithm. However, we do urge caution in the interpretation of the modified Bratton-Marshall test, because false-positive results are common for a variety of reasons, including exposure to benzodiazepines, which are commonly used in the treatment of neonatal seizures. Isolated Neonatal Seizures: Adenylosuccinase Deficiency is Another Cause!Pediatric NeurologyVol. 47Issue 3PreviewI read with great interest the very useful review “Isolated Neonatal Seizures: When to Suspect Inborn Errors of Metabolism” by Ficicioglu and Bearden [1]. I would like to add adenylosuccinase deficiency to their causes for suspicion. This genetic, mainly neurologic disorder of the de novo purine synthesis was first reported in 1984 [2]. Most of the approximately 60 reported patients demonstrate variable associations of moderate to severe psychomotor retardation, epilepsy after their first years, autistic features, and growth retardation [3]. Full-Text PDF

Patellar fracture: atypical presentation of parathyroid bone disease

A 21-year-old male was admitted to the orthopaedic and trauma department following a left knee injury. On examination, he appeared dehydrated and had left-sided knee pain. Radiological investigations revealed a left patellar fracture (Figure 1A); in addition, incidental distal femoral and proximal tibial bone cysts were present. Routine serum biochemistry revealed severe hypercalcaemia with a corrected calcium 4.03 mmol/l. Initial management of hypercalcaemia included 4 l/24 hr intravenous (IV) saline (from Day 1), 30 mg IV pamidronate (Day 2) and 40 mg iv furosemide (Day 4), with improvement in serum calcium (3.01 mmol/l …

Kawasaki's disease: An unusual presentation

Atypical presentations of Kawasaki's disease have been described in the form of intestinal pseudo-obstruction, tonsillitis, hemorrhagic serous effusions, thrombocytopenia, and non-fulfillment of all criteria for diagnosis of Kawasaki's disease. However, presentation of Kawasaki's disease with shock and need for ionotropic support have been rarely described. We present a 4-year-old girl with Kawasaki's disease who presented with anasarca, oliguria, shock, and presence of dilated coronary arteries within 5 days of fever and responded to intravenous immunoglobulin (IVIG) and inotropic support.